Management of parturients with Factor XI deficiency-10year case series and review of literature.

This is an article reviewing the management of pregnant women with factor XI (FXI) deficiency. Retrospective review of the electronic records of 67 pregnancies in 25 women with FXI deficiency over a ten-year period was undertaken. All women received care at St Mary's Tertiary Referral Obstetric/Haematology Clinic for some or all of their pregnancies. Outcome measures included antenatal complications, mode of delivery, anaesthesia provided and postpartum haemorrhage (PPH) and management required. A positive bleeding history was identified in 50% of women prior to pregnancy. Fifteen pregnancies (22%) ended in first trimester miscarriage; there was 1 termination of pregnancy. Two pregnancies were complicated by Antepartum haemorrhage. Of the remaining 51 pregnancies there were 50 live births - 2 preterm and 48 at term. There was one antenatal (34 weeks gestation) stillbirth of a growth restricted baby and one neonatal death secondary to severe prematurity (24 weeks gestation). Twenty -five babies delivered vaginally (20 spontaneous and 5 instrumental). The remaining 26 were delivered by Caesarean section (9 elective and 17 emergency). A sub-analysis of 22 operative deliveries was reviewed; this suggested that regional anaesthesia was safe in selected women with FXI deficiency - a selection that was based on FXI level/range, presence/absence of bleeding history and intended operative intervention.Solvent detergent treated Fresh Frozen Plasma (SD-FFP/Octaplas) and Tranexamic Acid (TXA) were given to those considered vulnerable -an individualised decision made by the multidisciplinary team in accordance with BCSH guidance. Primary PPH complicated 10/51 (15%) deliveries. The commonest cause of PPH was due to atony. Secondary PPH was only seen in only one case. Bleeding in women with FXI deficiency is highly variable and, whilst it does not directly correlate with Factor XI levels, provision of replacement therapy is required if FXI levels are <15 IU/dL as per BCSH guidance. Women with Factor XI levels >40 IU/dL are considered safe for regional anaesthesia following prophylactic FFP as suggested by sub group analysis. Treatment of women with rare bleeding disorders during pregnancy should be by a multidisciplinary team of specialists, to include Haematologist, Anaesthetist and Obstetrician, all of whom have an interest in bleeding disorders in pregnancy. Decisions should then be individualised, based on the presence/absence of a bleeding history and the third trimester FXI levels. Delivery does not have to be by Elective Caesarean. With appropriate care both operative vaginal delivery and regional anaesthesia can be facilitated.

Episiotomy: What angle do you cut to the midline?

OBJECTIVE: Although episiotomies are the commonest obstetric procedure performed the technique of performing one varies amongst obstetricians and midwives. The angle of episiotomy to the midline in particular has been shown to influence the risk of developing obstetric anal sphincter injuries (OASIS). In order to identify the differences in technique and to identify targets for training we undertook a practice survey of episiotomies to analyse the differences in technique between grades of obstetricians and midwives. STUDY DESIGN: A prospective practice survey of staff working on delivery suite in a tertiary referral unit with 9000 deliveries/year was conducted between 01/10/2014 to 01/03/2015. Each participant was provided with a pictoral representation of a perineum and a pair of standard episiotomy scissors and asked to perform an episiotomy as per their usual practice. The profession and grade of each participant was recorded along with information regarding the incision including the angle to the midline, length of incision and lateral starting distance from the midline. One way ANOVA (unrelated) was used to perform statistical analysis using IBM SPSS v23. RESULTS: 101 staff members participated in the practice survey including 63 midwives, 9 junior trainees, 15 senior trainees and 14 consultants. The mean angle of incision to the midline of episiotomies was 47°, 51°, 66° and 77° for midwives, junior trainees, senior trainees and consultants respectively. The mean angle of incision performed by midwives was significantly different to senior trainees (p>0.01) and consultants (p<0.01). 45% of all episiotomies undertaken by midwives were done at an angle <45° to the midline, compared to 7% by senior trainees and none by consultants. CONCLUSIONS: This study identified clear deficiencies in the performance of episiotomies amongst obstetric trainees and midwives. Both midwives and obstetric trainees need to improve their technique if episiotomies are going to influence the incidence of OASIS and, more importantly the development of faecal incontinence. These results should be used to inform future training programmes to reduce the risks of OASIS.

Fatigue and breathlessness in pregnancy: a rare and sinister cause.

We describe a rare but sinister presentation of fatigue and dyspnoea in a 39-year-old woman at 16 weeks gestation. Blood tests and bone marrow aspirate confirmed the diagnosis of multiple myeloma. The patient was managed expectantly during pregnancy with plasma exchange and blood transfusion. The pregnancy continued without event; labour was induced at 35 weeks gestation and a healthy female infant weighing 3100 g was delivered vaginally following a 2 h, 5 min labour. The patient subsequently underwent six cycles of cyclophosphamide, thalidomide and dexamethasone (CTD) chemotherapy followed by an autologous stem cell transplant (SCT) and reduced intensity conditioning matched unrelated donor (RIC MUD) transplant.

Systemic lupus erythematosus, pregnancy and carcinoma of the tongue.

We present a case which describes a 29-year-old woman with systemic lupus erythematosus who was treated aggressively with cytotoxic immunosuppression. Five years later and approximately 12 weeks pregnant, she is confirmed as having carcinoma of the tongue. Not wishing to consider termination of her pregnancy, she underwent surgical resection, which included partial glossectomy with microvascular reconstruction. Good oral function (speech and swallowing) was restored within 2 weeks. The pregnancy proceeded relatively uneventfully to 37 weeks gestation when proteinuric hypertension necessitated induction of labour. She remains well with no evidence of recurrence. This case highlights the options available in the treatment of carcinoma of the tongue during pregnancy together with the ethical considerations required, balanced against optimising maternal outcomes.

Predictors of poor perinatal outcome following maternal perception of reduced fetal movements--a prospective cohort study.

BACKGROUND: Maternal perception of reduced fetal movement (RFM) is associated with increased risk of stillbirth and fetal growth restriction (FGR). RFM is thought to represent fetal compensation to conserve energy due to insufficient oxygen and nutrient transfer resulting from placental insufficiency. OBJECTIVE: To identify predictors of poor perinatal outcome after maternal perception of reduced fetal movements (RFM). DESIGN: Prospective cohort study. METHODS: 305 women presenting with RFM after 28 weeks of gestation were recruited. Demographic factors and clinical history were recorded and ultrasound performed to assess fetal biometry, liquor volume and umbilical artery Doppler. A maternal serum sample was obtained for measurement of placentally-derived or modified proteins including: alpha fetoprotein (AFP), human chorionic gonadotrophin (hCG), human placental lactogen (hPL), ischaemia-modified albumin (IMA), pregnancy associated plasma protein A (PAPP-A) and progesterone. Factors related to poor perinatal outcome were determined by logistic regression. RESULTS: 22.1% of pregnancies ended in a poor perinatal outcome after RFM. The most common complication was small-for-gestational age infants. Pregnancy outcome after maternal perception of RFM was related to amount of fetal activity while being monitored, abnormal fetal heart rate trace, diastolic blood pressure, estimated fetal weight, liquor volume, serum hCG and hPL. Following multiple logistic regression abnormal fetal heart rate trace (Odds ratio 7.08, 95% Confidence Interval 1.31-38.18), (OR) diastolic blood pressure (OR 1.04 (95% CI 1.01-1.09), estimated fetal weight centile (OR 0.95, 95% CI 0.94-0.97) and log maternal serum hPL (OR 0.13, 95% CI 0.02-0.99) were independently related to pregnancy outcome. hPL was related to placental mass. CONCLUSION: Poor perinatal outcome after maternal perception of RFM is closely related to factors which are connected to placental dysfunction. Novel tests of placental function and associated fetal response may provide improved means to detect fetuses at greatest risk of poor perinatal outcome after RFM.

Haemoptysis in pregnancy caused by a well-differentiated fetal adenocarcinoma: a case report.

INTRODUCTION: Haemoptysis in pregnancy is frequently assumed to be caused by a pulmonary embolism. However, it can also be an indicator of serious pathology. CASE PRESENTATION: We report the case of a 27-year-old Caucasian woman who presented with haemoptysis in pregnancy that was discovered to be caused by a well-differentiated fetal adenocarcinoma of the lung. CONCLUSION: This case demonstrates the importance of establishing an accurate diagnosis when a pregnant woman presents with haemoptysis and that more serious pathology should be considered if the clinical symptoms persist and/or the presumed diagnosis of pulmonary embolism is not confirmed.

Endometrial adenocarcinoma: an analysis of treatment and outcome.

This study aims to review the survival and morbidity in patients treated for endometrial cancer, at a single centre and analyses the effects of co-morbidity on these outcomes. Case notes of all patients referred to the Christie Hospital with endometrial carcinoma from January 1, 1993 to December 31, 1995 (n=499) were reviewed. Twenty patients presented with recurrence and were not included in this analysis. Three hundred and seventy-five patients had previously undergone a total abdominal hysterectomy and bilateral salpingoophorectomy (+/- pelvic lymphadenectomy). Of these, 175 received adjuvant external beam radiotherapy (XRT) only, 49 received XRT and brachytherapy, 30 received brachytherapy alone and 121 patients had no further therapy. One hundred and four patients were referred for primary treatment. Radical radiotherapy was administered to 63 patients who were unfit for surgery, with 10 of these receiving XRT + brachytherapy and 53 receiving brachytherapy alone. Thirteen patients received palliative XRT and 28 supportive care only. The overall 5-year survival for those treated radically was 73.3%. There was no significant survival difference between patients who underwent surgery and adjuvant radiotherapy, in whatever form (p=0.115). Patients who did not undergo surgery did less well as a group, although there was no significant survival difference between those treated with combination therapy or brachytherapy alone (p=0.33). Survival was significantly associated with FIGO stage, tumour grade, age (especially those >75 years) and co-morbidity (ACE-27 score). Late morbidity occurred in 46 patients, with severe toxicity affecting 12 (3.8%). Toxicity was associated with ACE-27 score (p=0.0019), treatment dose and modality, with 50% (n=6) of severe toxicity seen in patients receiving adjuvant XRT + ICT. These data demonstrate that survival in patients with endometrial carcinoma treated radically remains good, with the stage and grade of tumour being significant factors for overall survival. The incidence of severe morbidity related to radiotherapy of any modality was 3.8%. A high co-morbidity (ACE-27) score was significantly associated with poorer survival (p<0.0055) and increased late treatment morbidity (p=0.0019).

Better life expectancy in women with BRCA2 compared with BRCA1 mutations is attributable to lower frequency and later onset of ovarian cancer.

PURPOSE: No formal assessment of life expectancy in women with BRCA1 and BRCA2 mutations in these genes has been reported previously. We have evaluated life expectancy using actuarial analysis and assessed the effect of breast and ovarian cancers on premature death in >1,000 BRCA1/2 carriers. METHODS: Families with pathogenic mutations in BRCA1 and BRCA2 have been ascertained in a 10-million population region of United Kingdom since 1996. Mutation carriers and their first-degree relatives were used in an analysis of breast and ovarian cancer incidence and mortality as well as to derive and compare an actuarial assessment of life expectancy. RESULTS: Six hundred twelve BRCA1 and 482 BRCA2 female mutation carriers were identified from 482 families. Life expectancy was significantly reduced for BRCA1 carriers compared with BRCA2 (P = 0.0002). This effect was attributable to an increased death rate from ovarian cancer (P = 0.04). Kaplan-Meier analysis revealed a better long-term survival from early-stage ovarian cancer in BRCA2 carriers but no significant differences in deaths from breast cancer or from women presenting with late-stage ovarian cancer. There was no other major contributing cause to death other than breast/ovarian cancer in BRCA1/2 female carriers. CONCLUSION: Interventions to reduce ovarian cancer incidence are likely to have a greater effect on life expectancy in BRCA1 compared with BRCA2 carriers.

Chronic pain and obstetric management of a patient with tuberous sclerosis.

Chronic nonmalignant pain is very disabling and carries a heavy financial strain on the individual and society as a whole. This case describes a woman with tuberous sclerosis, in her fourth pregnancy. Approximately 18 months prior to pregnancy, intractable left loin pain, thought to be secondary to hemorrhage within a tuberous lesion in the left kidney, had led to the siteing of an intrathecal morphine pump. The risks of system failure (dislodgement, dislocation), escalating dosage, infection, use in labor, and neonatal opioid withdrawal are all explored and discussed. While data are limited, with increasing use of intrathecal opioids for nonmalignant pain, such patients may be seen more regularly in obstetric clinics. With a multidisciplinary team approach, risks can be minimized and outcome for mother and baby optimized.

The lost intrauterine device: removal by hysterectomy.

A case of migration of an intrauterine device (IUD) deep within the endometrium, presenting as heavy vaginal bleeding and abdominal pain, is discussed. The IUD had been in situ for 31 years. Standard retrieval techniques failed and removal necessitated hysterectomy. The complications of 'lost IUDs' are discussed and the need to enquire about removal in all women on approaching the menopause is emphasised.

Egg donation--the donor's view: an aid to future recruitment.

The major stumbling block for egg donation lies in the recruitment of sufficient suitable donors. This study ascertained the views of egg donors in the UK by analysing 113 completed questionnaires that asked questions about demographics, stimulus to donate, support network available, ethics, the 'process' of donation and payment. Ideas for future recruitment were also sought. The mean age of donors was 31.7 years, and most donors were donating for the first time. Ninety-one per cent of donors were Caucasian and 93% had children of their own. Ninety-six (85%) donors felt fully supported in their decision to become an egg donor and 60 (53%) discussed their donation with their GP. Information regarding egg donation came from many sources. The main motivation to donate was a desire to help childless couples. Many respondents had themselves suffered, or knew of couples with, infertility. Eighty-three (73.5%) respondents felt that expenses alone should be paid to egg donors, and many expressed concerns that large financial incentives may attract the 'wrong women' to donate. Forty-nine (43%) respondents found the procedure painful or stressful in some way, although 95% had no regrets concerning their donation, and 72% would donate again. A common reason for donors not wishing to donate again was age restriction. Respondents were asked their opinion with regard to recruitment and the enthusiasm they expressed needs to be harnessed if the current shortcomings in available donors are to be overcome. Specific recommendations to achieve this are made.